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Clinical diagnosis and staging of cholangiocarcinoma

Nature Reviews Gastroenterology & Hepatology volume 8, pages 512–522 (2011)Cite this article

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Abstract

Cholangiocarcinoma is the most frequent biliary malignancy. It is difficult to diagnose owing to its anatomic location, growth patterns and lack of definite diagnostic criteria. Currently, cholangiocarcinoma is classified into the following types according to its anatomic location along the biliary tree: intrahepatic, perihilar or distal extrahepatic cholangiocarcinoma. These cholangiocarcinoma types differ in their biological behavior and management. The appropriate stratification of patients with regard to the anatomic location and stage of cholangiocarcinoma is a key determinate in their management. Staging systems can guide this stratification and provide prognostic information. In addition, staging systems are essential in order to compare and contrast the outcomes of different therapeutic approaches. A number of staging systems exist for cholangiocarcinoma—several early ones have been updated, and new ones are being developed. We discuss the emerging diagnostic criteria as well as the different staging systems for cholangiocarcinoma, and provide a critical appraisal regarding these advances in biliary tract malignancies.

Key Points

  • Cholangiocarcinoma is classified according to its anatomic location into three subtypes: intrahepatic, perihilar and distal extrahepatic cholangiocarcinoma

  • The diagnosis of intrahepatic cholangiocarcinoma requires histopathology and is a diagnosis of exclusion; a pathologic staging system for this cholangiocarcinoma subtype has been proposed

  • The diagnosis of perihilar cholangiocarcinoma is often made clinically, and is aided by cytologic fluorescent in situ hybridization studies; staging systems for this subtype of cholangiocarcinoma are still evolving

  • A diagnosis of distal extrahepatic cholangiocarcinoma can usually be confirmed by cytology; stage is highly dependent upon depth of invasion of surrounding structures

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Figure 1: Cholangiocarcinoma subtypes.
Figure 2: Intrahepatic cholangiocarcinoma growth types.
Figure 3: Histpathological features of mucin-producing cholangiocarcinoma and cholangiolocellular carcinoma with mixed features.
Figure 4
Figure 5

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Acknowledgements

This work was supported in part by grant DK59427 from the NIH (G. J. Gores), the Mayo Clinic Clinical Investigator Program (B. Blechacz) and the Mayo Foundation.

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Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, College of Medicine, Mayo Clinic, 200 First Street, Rochester, 55905, MN, USA

    Boris Blechacz & Gregory J. Gores

  2. Morphology and Molecular Pathology Section, Katholieke University Leuven, University Hospital Street Rafael, Minderbroederstraat 12, Leuven, B-3000, Belgium

    Mina Komuta & Tania Roskams

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  1. Boris Blechacz
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Contributions

B. Blechacz and G. J. Gores contributed to all aspects of this Review. M. Komuta contributed to the writing and editing; T. Roskams contributed to the discussion of content, writing and editing of this Review.

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Correspondence to Gregory J. Gores.

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Supplementary information

Supplementary Box 1

American Joint Cancer Committee/Union for International Cancer Control 7th edition TNM staging for intrahepatic cholangiocarcinoma (DOC 33 kb)

Supplementary Box 2

Liver Cancer Study Group of Japan (LCSGJ) staging system for intrahepatic cholangiocarcinoma (DOC 31 kb)

Supplementary Box 3

National Cancer Center in Japan (NCCJ) staging system for mass-forming intrahepatic cholangiocarcinoma (DOC 32 kb)

Supplementary Box 4

American Joint Cancer Committee/Union for International Cancer Control 7th edition TNM staging for perihilar cholangiocarcinoma (DOC 33 kb)

Supplementary Box 5

American Joint Cancer Committee/Union for International Cancer Control 7th edition TNM staging for distal extrahepatic cholangiocarcinoma (DOC 33 kb)

Supplementary Table 1

Memorial Sloan-Kettering Cancer Center staging system for hilar cholangiocarcinoma (DOC 38 kb)

Supplementary Table 2

Proposed clinical staging system for perihilar cholangiocarcinoma (DOC 41 kb)

Supplementary Table 3

Proposed staging system by Deoliveira et al. (DOC 80 kb)

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Blechacz, B., Komuta, M., Roskams, T. et al. Clinical diagnosis and staging of cholangiocarcinoma. Nat Rev Gastroenterol Hepatol 8, 512–522 (2011). https://doi.org/10.1038/nrgastro.2011.131

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